Overview

Definition:
-Hypertensive crisis (HTN-C) in children refers to a sustained and severe elevation in blood pressure (BP) that poses an immediate risk of target organ damage
-When secondary to underlying renal disease, it implies a direct link between kidney pathology and the acute rise in BP, often due to volume overload, activation of the renin-angiotensin-aldosterone system (RAAS), or impaired nitric oxide production.
Epidemiology:
-HTN-C in pediatrics is less common than in adults, with incidence varying based on the underlying cause
-Renal disease is a leading cause of secondary hypertension in children, contributing significantly to HTN-C presentations in EDs
-Conditions like acute glomerulonephritis, chronic kidney disease (CKD), and renovascular disease are prominent etiologies.
Clinical Significance:
-Prompt recognition and management of HTN-C secondary to renal disease are crucial to prevent irreversible end-organ damage, including encephalopathy, retinopathy, cardiac failure, and acute kidney injury progression
-This condition is a high-yield topic for DNB and NEET SS examinations due to its critical nature and complexity.

Clinical Presentation

Symptoms:
-Headache, often severe and throbbing
-Visual disturbances such as blurred vision or transient blindness
-Neurological symptoms including lethargy, confusion, seizures, or focal deficits
-Chest pain or shortness of breath suggestive of myocardial ischemia or pulmonary edema
-Nausea and vomiting
-Epistaxis or hematuria may be present if related to underlying renal pathology.
Signs:
-Markedly elevated systolic and diastolic blood pressure readings, often >95th percentile for age and height, or >120/80 mmHg in neonates
-Fundoscopic examination may reveal papilledema, hemorrhages, or exudates
-Cardiovascular findings can include a loud S2, gallop rhythm, or signs of heart failure
-Neurological signs may range from altered mental status to focal neurological deficits
-Peripheral edema may be present.
Diagnostic Criteria:
-A sustained BP reading of >95th percentile for age, sex, and height on at least two occasions
-Alternatively, a systolic BP >180 mmHg or diastolic BP >120 mmHg in infants and children, or >140/90 mmHg in neonates, particularly when associated with signs or symptoms of target organ dysfunction, constitutes a hypertensive urgency or emergency.

Diagnostic Approach

History Taking:
-Detailed history of previous BP readings, duration of hypertension, and associated symptoms
-Inquire about a history of renal disease, urinary tract infections, congenital anomalies, or family history of hypertension or renal disease
-Medication history, including use of nephrotoxic agents
-Recent onset of swelling, decreased urine output, or hematuria
-Presence of neurological, visual, or cardiac symptoms.
Physical Examination:
-Accurate serial BP measurements in both upper and lower extremities using appropriately sized cuffs
-Comprehensive neurological examination, including assessment of mental status, cranial nerves, and motor/sensory function
-Ophthalmoscopic examination for hypertensive retinopathy
-Cardiovascular assessment for murmurs, gallops, and signs of fluid overload
-Palpation for abdominal masses (e.g., Wilms tumor) or bruits suggestive of renovascular disease
-Examination for peripheral edema.
Investigations:
-Urinalysis: Proteinuria, hematuria, casts (e.g., red blood cell casts in glomerulonephritis)
-Serum creatinine and BUN: To assess renal function and detect AKI or CKD
-Electrolytes: Including sodium, potassium, and bicarbonate
-Complete blood count (CBC): To detect anemia or signs of infection
-Echocardiogram: To assess for left ventricular hypertrophy, diastolic dysfunction, or other cardiac sequelae
-Renal ultrasound: To evaluate kidney size, morphology, detect masses, or hydronephrosis
-Doppler ultrasound of renal arteries: To assess for renovascular stenosis
-ECG: To detect signs of acute ischemia or LVH
-Toxicology screen if drug-induced hypertension is suspected
-Plasma renin activity and aldosterone levels if primary hyperaldosteronism is considered.
Differential Diagnosis:
-Other causes of pediatric hypertension including essential hypertension, coarctation of the aorta, endocrine causes (pheochromocytoma, Cushing's syndrome), and iatrogenic causes (medications)
-Distinguishing between hypertensive urgency (severely elevated BP without target organ damage) and hypertensive emergency (severely elevated BP with acute target organ damage) is critical.

Management

Initial Management:
-Immediate BP reduction is the priority in hypertensive emergencies
-Gradual reduction is preferred to avoid hypotension and hypoperfusion, typically aiming for a 25% reduction in MAP within the first hour, followed by slower reduction over 2-24 hours
-Continuous BP monitoring via arterial line is ideal.
Medical Management:
-Intravenous (IV) antihypertensive agents are used
-For renal disease-related HTN-C, agents that do not significantly impair renal perfusion are preferred
-Common choices include: Labetalol (IV infusion or bolus), Nicardipine (IV infusion), or Fenoldopam (IV infusion, a dopamine-1 agonist with renal vasodilator effects)
-Hydralazine may be used cautiously but can cause reflex tachycardia and has variable bioavailability
-Sodium nitroprusside can be used for severe emergencies but requires careful monitoring due to cyanide toxicity risk
-Oral agents like nifedipine (short-acting) are generally avoided due to risk of precipitous BP drops.
Surgical Management:
-Surgical intervention is typically reserved for specific underlying renal pathologies causing hypertension, such as: surgical correction of renal artery stenosis (e.g., angioplasty with stenting or bypass), nephrectomy for unilateral renal lesions causing significant hypertension, or tumor resection (e.g., Wilms tumor, pheochromocytoma)
-These are usually not emergent procedures unless the HTN-C is directly attributable to an acute surgical complication.
Supportive Care:
-Fluid management is critical
-avoid fluid overload in patients with compromised renal function
-Strict fluid balance monitoring
-Continuous cardiac and neurological monitoring
-Management of associated complications like seizures with benzodiazepines
-Nutritional support, especially if oral intake is compromised.

Complications

Early Complications:
-Hypertensive encephalopathy leading to seizures, stroke, or coma
-Acute myocardial infarction or heart failure
-Aortic dissection
-Retinopathy with vision loss
-Acute kidney injury progression or irreversible renal damage
-Posterior reversible encephalopathy syndrome (PRES).
Late Complications:
-Chronic kidney disease progression
-End-stage renal disease requiring dialysis or transplantation
-Long-term cardiovascular sequelae including LVH and diastolic dysfunction
-Persistent visual impairment
-Cognitive deficits.
Prevention Strategies:
-Aggressive management of underlying renal disease
-Regular BP monitoring in children with known renal conditions
-Strict adherence to prescribed antihypertensive medications
-Education of caregivers on medication compliance and recognition of HTN-C symptoms
-Avoidance of nephrotoxic agents
-Prompt diagnosis and treatment of urinary tract infections and other causes of acute kidney injury.

Prognosis

Factors Affecting Prognosis:
-The severity and duration of the hypertensive crisis
-The extent of target organ damage at presentation
-The underlying cause of renal disease
-The promptness and efficacy of treatment
-The presence of comorbidities.
Outcomes:
-With prompt and appropriate management, HTN-C can be stabilized, preventing immediate life-threatening complications
-However, long-term prognosis is often dictated by the underlying renal disease, with many children requiring ongoing antihypertensive therapy and renal support
-Early intervention significantly improves outcomes and reduces the risk of long-term sequelae.
Follow Up:
-Close follow-up with pediatric nephrology is essential
-Regular BP monitoring, assessment of renal function (serum creatinine, BUN, urinalysis), and cardiac evaluation
-Management may require titrating antihypertensive medications, dietary modifications (low sodium), and consideration of renal replacement therapy if indicated
-Long-term surveillance for cardiovascular and renal complications is vital.

Key Points

Exam Focus:
-Remember the BP thresholds for hypertensive crisis in different pediatric age groups
-Prioritize rapid but controlled BP reduction in hypertensive emergencies
-Labetalol and nicardipine are first-line IV agents
-Fenoldopam offers renal benefits
-Suspect renal causes in children presenting with HTN-C, especially if accompanied by hematuria, proteinuria, or edema
-The management goal is not normalization, but controlled reduction to prevent end-organ damage.
Clinical Pearls:
-Always measure BP in both arms and legs in children with suspected HTN-C
-Consider a continuous infusion for titratable BP control over intermittent boluses
-Monitor for signs of hypoperfusion after BP lowering
-Involve pediatric nephrology early in management
-Treat the underlying cause of renal dysfunction vigorously alongside BP control.
Common Mistakes:
-Over-lowering BP too rapidly, leading to hypoperfusion and potential organ ischemia
-Underestimating the severity of hypertension or failing to recognize signs of end-organ damage
-Inadequate fluid management, especially in the presence of renal impairment
-Relying solely on oral medications in a hypertensive emergency
-Failing to investigate thoroughly for the underlying cause of renal disease.